Explain the fate of carbon skeleton and nitrogen group of amino acids. Explain the ways of transport of nitrogen from various parts of the body to the.

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Presentation on theme: "Explain the fate of carbon skeleton and nitrogen group of amino acids. Explain the ways of transport of nitrogen from various parts of the body to the."— Presentation transcript:

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Explain the fate of carbon skeleton and nitrogen group of amino acids. Explain the ways of transport of nitrogen from various parts of the body to the liver Describe the urea cycle and the enzymes involved in production of urea in the liver Define and classify Hyperammonemias. List the enzymes deficient in various hyperammonemias and its clinical features

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Sources of Amino Acids : Exogenous – Diet Endogenous – 1.Breakdown of muscle protein 2.Biosynthesis from intermediates of citric acid cycle. Utilization of Amino acids: Synthesis of New proteins Formation of Nucleotides Formations of Porphyrins and Catecholamines Production of energy and Ammonia.

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1) Reutilization: Glutamate and Glutamine are involved in recycling of amino acids. Glutamate + AmmoniaGlutamine Glutamine Synthase ATPADP They are secreted by the peripheral tissues in form of glutamine which is taken up by hepatocytes where the NH3 is re-used for amino acid and nucleotide synthesis Glutamine Glutamate + Ammonia Glutaminase

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Two important reactions are involved in fixing ammonia back to amino acids: 1.Reductive Amination: 2.Amino Transferases: All non-essential amino acids except for tyrosine and cysteine are derived and are dependent on transamination from glutamate.

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MCQ 1 Select the CORRECT answer. The first reaction in the degradation of the majority of common amino –acids involves participation of : A.NAD + B.Pyridoxal Phosphate C.Thiamine Pyrophosphate(TPP) D.FAD E.NAD and TPP

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MCQ 2 After thorough investigations a man is diagnosed with orotic aciduria. To find out the cause of orotic aciduria which of the following investigations will you prefer? A. ALP levels B. vitamin b12 assay C. FIGLU excretion assay D. Peripheral smear E. serum bilirubin